Outcomes following the Kawashima procedure for single-ventricle palliation in left atrial isomerism

Anne Vollebregt, Kuberan Pushparajah, Maleeha Rizvi, Andreas Hoschtitzky, David Anderson, Conal Austin, Shane M. Tibby, John Simpson*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

16 Citations (Scopus)


Objectives: Patients with left atrial isomerism and interrupted inferior vena cava palliated with a superior cavopulmonary connection or Kawashima procedure (KP) have a high incidence of developing pulmonary arteriovenous malformations. The necessity for hepatic vein redirection (HVR) and its timing remains a controversy. We aimed to assess the clinical outcome of patients with left atrial isomerism following a KP. The main end points were death, requirement for HVR and the impact of HVR on oxygen saturation. Methods: Retrospective review of 21 patients with a diagnosis of left atrial isomerism, interruption of the inferior vena cava and single-ventricle physiology managed with a KP at a single centre between January 1990 and March 2010. Results: Twenty-one patients had a KP, with 12 subsequently undergoing HVR. There was relatively a constant monthly decrement in the proportion of patients who were free from death or HVR up until 60 months following the KP, with a dramatic increase in the hazard after this time. The Cox proportional hazards regression model demonstrated a reduced early risk for HVR or death in patients who underwent pulmonary artery banding versus arterial shunt as the primary procedure (hazard ratio: 0.10; P = 0.01), and an increased risk with bilateral superior vena cavas (SVCs) (hazard ratio: 3.4; P = 0.04) and age at KP (hazard ratio: 1.02 per month increase in age at KP; P = 0.02). HVR mortality was relatively high with 3 of 12 patients dying in the early postoperative period with profound cyanosis. The timing of HVR after the KP did not influence the postoperative rate of increase in oxygen saturation. Conclusion: These findings confirm that the majority of patients who undergo a KP will require HVR. Patients who are older at the time of the KP or having an initial arterial shunt or bilateral SVCs are at higher risk of HVR or death. The relatively high mortality at HVR was characterized by severe postoperative cyanosis.

Original languageEnglish
Pages (from-to)574-579
Number of pages6
JournalEuropean Journal of Cardio-thoracic Surgery
Issue number3
Publication statusPublished - Mar 2012
Externally publishedYes

Bibliographical note

Funding Information:
J.S. and S.M.T. acknowledge financial support form the department of health via the National Institute for Health Research Comprehensive Biomedical Research Centre award to Guys and St Thomas’ Hospital NHS Foundation Trust in partnership with King’s College London and King’s College Hospital NHS Foundation Trust.


  • Hepatic vein redirection
  • Interrupted inferior vena cava
  • Isomerism
  • Kawashima
  • Pulmonary arteriovenous malformations


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